The impact of 18 F-FDG PET on initial staging and therapy planning of pediatric soft-tissue sarcoma patients
Conclusion18F-FDG PET proved to be a valuable tool for precise initial staging of pediatric soft-tissue sarcoma patients, especially in detecting lymph node metastasis, and could be included in their initial work-up. Given the relative rarity and heterogeneity of this group of tumors, additional investigations are required to definitely establish a role for18F-FDG PET in the initial staging and therapy planning of soft-tissue sarcoma in the pediatric population.
AbstractWhile sarcomas account for approximately 1% of malignant tumors of adults, they are particularly more common in children and adolescents affected by cancer. In contrast to malignancies that occur in later stages of life, childhood tumors, including sarcoma, are characterized by a striking paucity of somatic mutations. However, entity-defining fusion oncogenes acting as the main oncogenic driver mutations are frequently found in pediatric bone and soft-tissue sarcomas such as Ewing sarcoma (EWSR1-FLI1), alveolar rhabdomyosarcoma (PAX3/7-FOXO1), and synovial sarcoma (SS18-SSX1/2/4). Since strong oncogene-dependency h...
This article summarizes the clinical and imaging features of these rare tumors and describes in detail the three most common histological types of NRSTSs encountered in children — synovial sarcoma, malignant peripheral nerve sheath tumor and infantile fibrosarcoma. The author discusses the role of non-cross-sectional and cross-sectional imaging.
ConclusionsSurvival of sarcoma-SMN patients is worse than sarcoma-FMN patients. Although survival and tumor characteristics appear similar for breast-SMN and breast-FMN patients, treatment differs; breast-SMN patients less often receive breast-conserving therapy. Larger studies are necessary to substantiate these exploratory findings.
Authors: Krawczyk MA, Karpinsky G, Izycka-Swieszewska E, Gabrych A, Kunc M, Fatyga A, Garstka M, Styczewska M, Sokolewicz EM, Szlagatys-Sidorkiewicz A, Kazanowska B, Bien E Abstract BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is rare, aggressive soft tissue sarcoma which may affect children. OBJECTIVE: We aimed to assess prognostic significance of immunohistochemical (IHC) markers, osteopontin, fibronectin, survivin, cyclin D1 and p53, in pediatric MPNST. METHODS: A total of 26 pediatric MPNST patients were enrolled in the current study with a median follow-up of 51 months. IHC staining usin...
ConclusionsThis study suggests that the combined exposure to PARPi and IR might display a role in the treatment of RMS tumours compared with single-agent exposure, since stronger cytotoxic effects are induced, and compensatory survival mechanisms are prevented.
We report the case of a rhabdomyosarcoma, initially diagnosed as a dental abscess, in the mandible of a 7-year-old girl. Clinical examination revealed an expansive mass, 12 cm in diameter, in the body of the right jaw. On incisional biopsy, this was identified as a malignant neoplasm of low differentiation suggestive of sarcoma, requiring differential diagnosis between Ewing sarcoma and rhabdomyosarcoma.
ConclusionBased on the longstanding registration system, the childhood cancer incidence rates were similar to those observed in other Latin American countries. While a degree of under-ascertainment of cases cannot be excluded, the markedly high leukemia rates, in particular of the lymphoid sub-type deserves further study in this population.
ConclusionThe cumulative incidence of secondary cancer varied among primary cancers. The primary cancer was closely associated with the secondary cancer but stem cell transplantation was a common risk factor for secondary cancers among CCSs.
Abstract PURPOSE: Alveolar rhabdomyosarcoma (aRMS) is a childhood soft tissue sarcoma driven by the signature PAX3-FOXO1 (P3F) fusion gene. 5-year survival for aRMS is
Purpose of review Germline pathogenic TP53 mutation may predispose to multiple cancers but penetrance and cancer patterns remain incompletely documented. We have analyzed international agency for research on cancer TP53 database to reevaluate age and variant-dependent tumor patterns. Recent findings Genome-wide studies suggest that germline variants are more frequent than estimated prevalence of Li–Fraumeni syndrome (LFS), suggesting that many carriers of potentially pathogenic mutations may not develop the syndrome. Carriers of a germline TP53 mutation who are detected in a clinical context have a penetrance of...